Growth deficits in children with sickle cell disease

Growth deficits are common in children with sickle cell disease. Few prospective studies are available and the pathophysiologic basis for the impaired growth is not clearly understood. Our objectives were to collect data on anthropomorphic measurements of children with sickle cell disease prospectively followed for 1 year and to correlate them with hematologic data.

One hundred children <8 years of age (73 with homozygous SS sickle cell anemia [HbSS] and 27 with hemoglobinopathy SC [HbSC]) were included. Standardized Z scores of weight for age (waz), height for age (haz), and weight for height (whz) were compared to the National Center for Health Statistics (NCHS) reference population.

At study entry, the means (standard deviation [SD]) of waz, haz, and whz were -0.69 (1.06), -0.65 (1.11), and -0.32 (1.00), respectively. After 1 year of study, children with HbSS presented a significant decrease in waz (p = 0.01) and whz (p = 0.02); the decrease in haz was not statistically significant (p = 0.48). The effect was similar for children older or younger than 24 months of age. The decrease in waz and whz was significant for boys but not for girls. After 1 year of follow-up, lower mean waz scores were observed among patients with lower hemoglobin concentration and higher reticulocyte count (p = 0.03 and p = 0.08). Hemoglobin concentration was higher in girls. The anthropomorphic measurements did not deteriorate significantly in children with HbSC.

Growth deficits may be demonstrable in children with HbSS, even during a short period of observation. Fast red blood cell turnover may be partially responsible for the observed effect.